ESPN 51th Annual Meeting

ESPN 2018


 
Enhanced intrinsic skin ageing in nephropathic cystinosis assessed by High-Definition Optical Coherence Tomography
KOENRAAD VEYS 1 MOHAMED ELMONEM 1 FRANS DHAENENS 2 MARIA VAN DYCK 3 MIRIAN JANSSEN 4 MARLIES CORNELISSEN 5 KATHARINA HOHENFELLNER 6 AHMED REDA 1 PASCALE QUATRESOOZ 7 BERT VAN DEN HEUVEL 1 MARC BOONE 8 ELENA LEVTCHENKO 3

1- KU LEUVEN, BELGIUM, DEPARTMENT OF DEVELOPMENT AND REGENERATION
2- AGFA HEALTHCARE, MORTSEL, BELGIUM
3- DEPARTMENT OF PEDIATRICS, UZ LEUVEN, BELGIUM
4- RADBOUD UNIVERSITY MEDICAL CENTER, NIJMEGEN, THE NETHERLANDS, DEPARTMENT OF INTERNAL MEDICINE
5- RADBOUD UNIVERSITY MEDICAL CENTER, NIJMEGEN, THE NETHERLANDS, DEPARTMENT OF PEDIATRIC NEPHROLOGY
6- SUDOSTBAEYERN KLINIKEN, DEPARTMENT OF PEDIATRICS, TRAUNSTEIN, GERMANY
7- CENTRE HOSPITALIER UNIVERSITAIRE (CHU) DE LIèGE, DEPARTMENT OF PATHOLOGY, LIèGE, BELGIUM
8- HôPITAL ERASME, UNIVERSITé LIBRE DE BRUXELLES (ULB), BRUSSELS, BELGIUM
 
Introduction:

In nephropathic cystinosis, dermal cystine crystal accumulation and subtle clinical signs of premature skin ageing have been reported. Recently, reflectance confocal microscopy (RCM), has been applied for semi-quantification of dermal cystine crystal deposits. High-Definition Optical Coherence Tomography (HD-OCT, Agfa Healthcare, Belgium) is a novel, non-invasive optical imaging technique, offering the advantage of real-time in vivo visualization of the skin up to a depth of 570um. In this study, we aimed to investigate cutaneous manifestations of nephropathic cystinosis by use of HD-OCT, and to explore potential correlations with clinical disease severity and compliance with cysteamine treatment.

Material and methods:

HD-OCT imaging was performed at the inner side of the right upper arm in 18 nephropathic cystinosis patients, of whom 11 patients were prior to kidney transplantation, and 54 age & sex-matched healthy controls. HD-OCT images were analyzed by a method adapted from Boone et al, for epidermal and papillary dermis thickness, and several of its optical characteristics.

Results:

After validation of our method of HD-OCT image analysis, we demonstrated a significant reduction of epidermal and papillary dermis thickness in nephropathic cystinosis patients. More importantly, this effect was already present in adolescent patients prior to kidney transplantation. However, the relative attenuation factor of the papillary dermis showed no significant difference between cystinotic patients and controls, indicating that dermal cystine crystal deposition cannot be quantified by HD-OCT. No correlation was found between epidermal & papillary dermis thinning and age, kidney function (eGFR) or average leucocyte cystine level .

Conclusions:

In adolescent nephropathic cystinosis patients prior to kidney transplantation, thinning of epidermis and papillary dermis of non-sunexposed skin was demonstrated, suggesting an enhanced intrinsic skin ageing. Hence, HD-OCT and RCM can be regarded as complimentary techniques for the assessment of cutaneous manifestations of nephropathic cystinosis.